Lymphocytoma cutis - case report

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An Bras Dermatol. 2013;88(6 Suppl 1):128-31.

Lymphocytoma cutis - Case report

*

Linfocitoma cutis - Relato de caso

Ederson Valei Lopes de Oliveira

1

_{Giovana Bachega Badiale}

2

_{Margarida M. F. S. Moraes}

3

DOI: http://dx.doi.org/10.1590/abd1806-4841.20132320

Abstract:We describe a clinical case involving a 62-year-old white male, diagnosed with lymphocytoma cutis

(Spiegler-Fendt sarcoid) in the cephalic segment. The diagnosis was carried out by pathological study and con-firmed by immunohistochemical panel: evidence of polyclonality. Phototherapy sessions were suggested as treat-ment (13 PUVA sessions, with an accumulated dose of 58.65 J/cm² ). The improvetreat-ment was partial. Thus, infil-tration of triamcinolone was opted for (one intralesional infilinfil-tration every 3 weeks). After 5 sessions, satisfacto-ry improvement was observed: regression of nearly all the lesions.

Keywords: Immunohistochemistry; Lymphoma; Pseudolymphoma; Triamcinolone acetonide

Resumo: Relatamos um caso de um paciente de 62 anos, branco e com diagnóstico de linfocitoma cutis (Sarcoide de Spigler-Fendt) em segmento cefálico. O diagnóstico foi confirmado pelo histopatológico e reafirmado pelo pai-nel imuno-histoquímico: evidência de policlonalidade. Sessões de fototerapia foram propostas como tratamento: 13 sessões de PUVA cuja dose acumulada foi de 58.65 J/cm2); a melhora foi parcial. Optado, então, por infiltra-ções de triancinolona (uma infiltração intralesional a cada 3 semanas). Na quinta sessão, satisfatória melhora já podia ser evidenciada: regressão de quase todas as lesões.

Palavras-chave: Imunoistoquímica; Linfoma; Pseudolinfoma; Triancinolona acetonida

Received on 28.11.2012.

Approved by the Advisory Board and accepted for publication on 17.01.2013. * Study carried out at the Clínica Endoderm – Ribeirão Preto (SP), Brazil.

Conflict of interest: None Financial funding: None

1 _{Dermatologist. Private clinic – Ribeirão Preto (SP), Brazil.}

2 _{Pathology specialist, qualified at the Medical School of the University of de São Paulo, Ribeirão Preto (FMRP USP). Ribeirão Preto (SP), Brazil.}

3 _{Pathologist. Master’s in pathological anatomy. Training Doctor in the Pathological Anatomy department of the Medical School of the University of São Paulo,}

s

INTRODUCTION

Lymphocytoma cutis, or Spiegler-Fendt sarcoid, is classed as one of the pseudolymphonas,1 referring to inflammatory disorders in which the accumulation of lymphocytes on the skin resemble, clinically and histopathologically, cutaneous lymphomas.1-3

In order to obtain an accurate diagnosis, careful clinical evaluation is needed, as well as histopatholog-ical and immunohistochemhistopatholog-ical exams.1-3

Here, we describe a case involving a patient over 60, with lesions distributed across upper parts of the head, whose final diagnosis was lymphocytoma cutis.

Despite the improvement in the lesions follow-ing corticoid infiltrations, the patient remains under clinical observation, since, in some cases, the condi-tion can evolve into cutaneous lymphoma.

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CASE REPORT

White male, 62, suffering for a year from lesions in the skin in the frontal, zygomatic lateral and tempo-ral anterior (both bilatetempo-rally), regions. There was no itching but he experienced occasional pain to local palpation. He sought help from doctors, who pre-scribed ketoconazole shampoo and antihistamines, among other medications, but to no avail.

The patient denied being a smoker or a heavy drinker, and was taking omeprazole to control the reflux disease.

The physical exam revealed macules, papules, nodules and plaques. The lesions presented in an ery-thematous-violaceous color, and others in erythema-tous-pink. There was no scaling (Figures 1 and 2).

Further diagnoses included sarcoidosis, pseudolymphoma and cutaneous lymphoma. Other

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Lymphocytoma cutis - Case report 129

more unlikely hypotheses were: luminal eruption, rosacea and erythematous lupus, borreliosis.

Serum exams revealed no alterations. The serum calcium dosage was applied, as a hypercal-cemia suggested the diagnosis of sarcoidosis.

The ANF was non-reactive, suggesting, in prin-ciple, that the condition was not autoimmune.

There were non-reactive IgM and IgG antiborre-lia antibodies, eliminating the possibility of borreliosis.

A chest x-ray revealed no pulmonary alter-ations (which thus reduced the chances of sarcoido-sis). An ultrasound of the abdomen showed the pres-ence of light hepatic steatosis.

The skin biopsy revealed hydropic degenera-tion of the basal layer. In the dermis, there was chron-ic and nonspecifchron-ic inflammatory infiltrate, with a pre-dominance of lymphocytes in the reticular dermis and the formation of lymphoid follicles: germinative cen-ters (Figure 3).

The immunohistocemistry confirmed polyclon-al lymphoid infiltrate: lymphocytes B-CD20 and lym-phocytes T-CD3 (Figures 4 and 5).

The immunohistochemical analysis was also complemented by the BCL2, CD4, CD7 and CD8, markers: thus reaffirming the polyclonality.

The initial treatment suggested was photother-apy: 13 PUVA sessions were carried out in a period of 2 months, with a total accumulated dose of 58.65 J/cm2_{(8-MOP administered twice a week with a dose} of 0.65mg/kg). There was some improvement, though the patient could not continue with this treatment.

FIGURE1: Presence of erythematous-viola-ceous and eryt-hematous-pink macules, papules and nodules FIGURE2: Macules, papules and plaques. Some are erythematous-viola-ceous and others are erythema-tous-pink. There are no scaly lesions

FIGURE3: Skin fragment showing inflammatory infiltrate in the dermis. Predominance of lymphocytes in the reticular dermis. Formation of lymphoid follicles: germinative centers. (HE, 100x)

FIGURE4: Immunohistochemistry: CD 20: B lymphocytes

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Hence, the chosen treatment was infiltrations of triamcinolone acetonide 40mg/ml, every 10-15 days. Clinical improvement was noted during the 5th ses-sion.(Figure 6)

DISCUSSION

Lymphocytoma cutis, or Spiegler-Fendt sar-coid, can be understood as a lymphoreticular and hyperplasic reaction occurring in the dermis.1-2,4_{It is a}

pseudolymphoma of the B cells.4-5

Many possible triggering factors have been identified: acupuncture, earrings, piercings, tattoos, phenytoin, vaccinations, infections from the herpes simplex virus and zoster, insect bites, and infection by the bacterium Borrelia burgdorferi.1-2,5_{However, in most}

cases, the trigger seems to be idiopathic.2,5

It is a rare disease, generally affecting young adults (under the age of 40), with a predilection for females (3:1).2,4-7 _{However, cases involving different}

age groups have been reported.3 _{It affects}

predomi-nantly caucasians.5

FIGURE6: Regression of lesions after infil-trations of triam-cinolone

Clinically, lesions present as papules and/or nodules that can be shiny.1-5,8_{They are not scaly and can}

be skin-colored, erythematous, brownish or viola-ceous.1,4,5_{Itching can occur. In the literature, it is}

com-mon to find descriptions of lesions with softened con-sistency.1,5_{In the case under discussion, the consistency}

of the lesions was similar to that of non-affected skin. The localized form accounts for 70% of cases, occurring predominantly on the face or upper limbs. The disseminated form is rarer, found mostly in elder-ly people, affecting the midsection and extremities, in addition to the face.5-6,8_{The clinical condition in which}

there is dissemination of lesions is termed miliary lymphocytoma cutis.2,5

Differential diagnoses include: cutaneous lym-phoma, sarcoidosis, erythematous lupus, angiolym-phoid hyperplasia, luminal eruption and rosacea.1-3,5

Histopathologically, it is characterized by foci of lymphocytes in the dermis, permeated by histio-cytes, bringing about a follicular arrangement that is translated by B cells bordered by T cells, that is to say, the germinative centers. With lymphomas, there are no germinative centers, except in cases of malignant lymphomas of the giant follicular type.3

The dermis can be separated from the epider-mis by a thin area of collagen.2,5

Immunohistochemistry reveals the polyclonali-ty (presence of B lymphocytes and T lymphocytes).3,5

Therapeutics options include systemic corti-coids, corticoid infiltrations, superficial radiotherapy, PUVA, cryosurgery, hydroxychloroquine, surgery, photodynamic therapy.1,2,4-7,9,10 _{When the}

lymphocy-toma cutis is associated with borreliosis, treatment is tailored to this condition.

Follow-up of patients is crucial, as the condition can evolve into cutaneous lymphoma.2,4 q

ACKNOWLEDGEMENTS

The authors are grateful for the valuable contri-butions of: Dr. Daniela Chaves Tarquínio, who con-ducted the phototherapy sessions; and Dr. Mariângela Esther Alencar Marques, who helped in the histopathological study.

An Bras Dermatol. 2013;88(6 Suppl 1):128-31. 130 Oliveira EVL, Badiale GB, Moraes MMFS

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Lymphocytoma cutis - Case report 131

How to cite this article: Oliveira EVL, Badiale GB, Moraes MMFS. Lymphocytoma cútis - Case report. An Bras Dermatol. 2013;88(6 Suppl 1):S128-31.

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